今日の臨床サポート

皮膚リンパ腫

著者: 濱田利久 国際医療福祉大学成田病院皮膚科

監修: 戸倉新樹 掛川市・袋井市病院企業団立 中東遠総合医療センター 参与/浜松医科大学 名誉教授

著者校正/監修レビュー済:2022/03/30
参考ガイドライン:
  1. 日本皮膚科学会:皮膚悪性腫瘍ガイドライン第3版 皮膚リンパ腫診療ガイドライン2020
患者向け説明資料

概要・推奨   

  1. ステロイド外用/紫外線療法に対して治療抵抗性の早期菌状息肉症(病期ⅡAまで)に対し、追加治療としてレチノイドはインターフェロン-γよりも推奨できるか?
推奨:治療抵抗性の早期菌状息肉症(病期ⅡAまで)に対し、追加治療としてまずはインターフェロン-γを提案する.GRADE 2D(推奨の強さ:弱い推奨、エビデンスの確信性:非常に低)
  1. 進行期菌状息肉症(病期ⅡB以上)に対する全身治療として、経口エトポシドはインターフェロン-γ、レチノイド、ボリノスタットよりも推奨できるか?
推奨:進行期菌状息肉症(病期ⅡB以上)に対する全身治療として経口エトポシドを第一選択としないことを提案する.GRADE 2D(推奨の強さ:弱い推奨、エビデンスの確信性:非常に低)
  1. 菌状息肉症の局面性・腫瘤性病変に対して、全身または病変局所の低線量(総線量4~20 Gy)電子線照射は、従来線量(総線量20~40 Gy)と比べて推奨できるか?
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薬剤監修について:
オーダー内の薬剤用量は日本医科大学付属病院 薬剤部 部長 伊勢雄也 以下、林太祐、渡邉裕次、井ノ口岳洋、梅田将光による疑義照会のプロセスを実施、疑義照会の対象については著者の方による再確認を実施しております。
※薬剤中分類、用法、同効薬、診療報酬は、エルゼビアが独自に作成した薬剤情報であり、
著者により作成された情報ではありません。
尚、用法は添付文書より、同効薬は、薬剤師監修のもとで作成しております。
※薬剤情報の(適外/適内/⽤量内/⽤量外/㊜)等の表記は、エルゼビアジャパン編集部によって記載日時にレセプトチェックソフトなどで確認し作成しております。ただし、これらの記載は、実際の保険適用の査定において保険適用及び保険適用外と判断されることを保証するものではありません。また、検査薬、輸液、血液製剤、全身麻酔薬、抗癌剤等の薬剤は保険適用の記載の一部を割愛させていただいています。
(詳細はこちらを参照)
著者のCOI(Conflicts of Interest)開示:
濱田利久 : 特に申告事項無し[2021年]
監修:戸倉新樹 : 講演料(田辺三菱,サノフィ,マルホ,協和キリン),研究費・助成金など(ノバルティス,レオファーマ)[2021年]

改訂のポイント:
  1. 『皮膚悪性腫瘍ガイドライン第3版』に基づき、全面的に改訂を行った。

病態・疫学・診察

疾患情報(疫学・病態)  
ポイント:
  1. 皮膚リンパ腫は、皮膚に原発もしくは皮膚と親和性が高い節外性リンパ腫の一群。
  1. T/NK細胞リンパ腫とB細胞リンパ腫に分類され、T/NK細胞リンパ腫が病型数、罹患患者数とも多いのが特徴。
  1. ⽶国の疫学調査では、年間100万⼈に12〜14⼈程度の罹患率[2]、わが国の⽪膚科専⾨施設が参加するレジストリでは、年間400人程度の罹患者数[3]
  1. 罹患者数の約半数は菌状息⾁症でもっともポピュラー[3][4]
  1. 菌状息⾁症・セザリー症候群は、臨床病期分類による生命予後の推定が可能[5][6][7]
  1. その他の皮膚リンパ腫においては、病型自体が一番の生命予後関連因子[8]
 
 
成人T細胞白血病リンパ腫の皮疹タイプと生命予後

a:局面型
b:結節腫瘤型
c:紅皮症型
d:紫斑型
Sawada らによると、皮疹を局面型・結節腫瘤型・紅皮症型・紫斑型に分けると、それぞれの生存期間中央値は、114.9カ月、17.3カ月、3.0カ月、4.4カ月で、紅皮症型と紫斑型の生命予後がより不良であった。また各予後関連因子で多変量解析すると、結節腫瘤型と紅皮症型が独立した予後不良因子であった[10]

出典

img1:  著者提供
 
 
 
菌状息肉症:
  1. 皮膚に初発し、典型的には、斑、局面、腫瘤と進展する末梢性リンパ腫。
  1. 病理組織学的に、表皮向性を伴ったCD4(まれにCD8)陽性T細胞が浸潤する。
  1. 病初期は皮膚に限局し、進行は非常に緩徐のことも多い。
  1. アトピー性皮膚炎や乾癬に酷似することもあり、これら良性疾患との鑑別が必要。
 
菌状息肉症の臨床像と病理組織像

斑(紅斑)、局面から進展すると腫瘤を形成し得る。腫瘤は潰瘍形成をしばしば伴って強い疼痛を合併し得る。病理組織学的にはやや大型で脳回転状の核を有する異型リンパ球が表皮内に侵入し集塊(ポートリエ微小膿瘍)を形成するのが特徴で、これらは通常CD3陽性、CD4陽性のT細胞のphenotype を有している。

出典

img1:  著者提供
 
 
 
セザリー症候群:
  1. 紅皮症、リンパ節腫脹、末梢血の異常リンパ球出現(白血化)を3主徴とする。
  1. 菌状息肉症から進展する症例もみられるために一疾患群として取り扱われる。
  1. 疾患特異的5年生存率は3割程度とされ、不良である。
  1. 白血化の基準は、クローン性増殖を伴って(T細胞受容体遺伝子にクロナリティあり)、異常リンパ球が末梢血中に1,000個/μL以上出現。形態学的異常が目立たない場合、末梢血のフローサイトメトリーでCD4/CD8≧10、CD4+CD7-細胞≧40%、あるいはCD4+CD26-細胞≧30%のどれかを満たせばセザリー細胞と解釈できる。
 
セザリー症候群の臨床像・血液像

セザリー症候群は紅皮症・リンパ節腫脹・末梢血へのセザリー細胞の出現(白血化)を3徴候とする。フローサイトメトリーで、CD4/8比; 29.3、CD4+CD7-分画; 84%、CD4+CD26-分画; 83.6% と腫瘍性と考えられるCD4+T細胞分画が末梢血中に増加している。

出典

img1:  著者提供
 
 
問診・診察のポイント  
  1. 詳細な現病歴が重要である。典型的な菌状息肉症では斑で初発し進行に従って局面・腫瘤を形成するが、腫瘤期でもこれらが混在する。

今なら12か月分の料金で13ヶ月利用できます(個人契約、期間限定キャンペーン)

6月30日(木)までにお申込みいただくと、通常12ヵ月の使用期間が1ヶ月延長となり、13ヵ月ご利用いただけるようになります。

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文献 

著者: Porcia T Bradford, Susan S Devesa, William F Anderson, Jorge R Toro
雑誌名: Blood. 2009 May 21;113(21):5064-73. doi: 10.1182/blood-2008-10-184168. Epub 2009 Mar 11.
Abstract/Text There have been no prior large population-based studies focusing on cutaneous lymphomas (CL) in the United States. Using the Surveillance, Epidemiology and End Results (SEER) program data, we analyzed age-adjusted CL incidence rates (IRs) and survival rates by sex and race/ethnicity. There were 3884 CLs diagnosed during 2001-2005. Cutaneous T-cell lymphomas (CTCLs) accounted for 71% (age-adjusted incidence rate [IR] = 7.7/1 000 000 person-years), whereas cutaneous B-cell lymphomas(CBCLs) accounted for 29% (IR = 3.1/1 000 000 person-years). Males had a statistically significant higher IR of CL than females (14.0 vs 8.2/1 000 000 person-years, respectively; male-female IR ratio [M/F IRR] = 1.72; P < .001). CL IRs were highest among blacks and non-Hispanic whites (both 11.5/1 000 000 person-years), followed by Hispanic whites (7.9) and Asian/Pacific Islanders (7.1). The CTCL IR was highest among blacks (10.0/1 000 000 person-years), whereas the CBCL IR was highest among non-Hispanic whites (3.5). Over the past 25 years, the CL IR increased from 5.0/1 000 000 person-years during 1980-1982 to 14.3 during 2001-2003. During 2004-2005, the CL IR was 12.7. This recent apparent change could be incomplete case ascertainment or potential leveling off of IRs. CLs rates vary markedly by race and sex, supporting the notion that they represent distinct disease entities.

PMID 19279331  Blood. 2009 May 21;113(21):5064-73. doi: 10.1182/blood-・・・
著者: Toshihisa Hamada, Keiji Iwatsuki
雑誌名: J Dermatol. 2014 Jan;41(1):3-10. doi: 10.1111/1346-8138.12299.
Abstract/Text Types of cutaneous lymphoma (CL) and their incidences may vary among geographic areas or ethnic groups. The present study aimed to investigate the incidences of various CL in Japan, using epidemiological data from a nationwide registration system for CL. Between 2007 and 2011, 1733 new patients with CL were registered from over 600 dermatological institutes in Japan. The 1733 patients registered included 1485 (85.7%) patients with mature T- and natural killer (NK)-cell neoplasms, 224 (12.9%) with B-cell neoplasms and 24 (1.4%) with blastic plasmacytoid dendritic cell neoplasm. Mycosis fungoides (MF) is the most common CL subtype in the present study (750 patients, 43.3%). The proportion of MF patients with early-stage disease was 73%, similar to that of previous studies from other cohorts. The incidence rates of adult T-cell leukemia/lymphoma and extranodal NK/T-cell lymphoma, nasal type were 16.7% and 2.0%, respectively, which may account for the higher incidence of mature T- and NK-cell neoplasms in Japan, as compared with that in the USA and Europe. A male predominance was observed in most types of CL, except for several CL subtypes such as subcutaneous panniculitis-like T-cell lymphoma.

© 2014 Japanese Dermatological Association.
PMID 24438138  J Dermatol. 2014 Jan;41(1):3-10. doi: 10.1111/1346-8138・・・
著者: Kazuyasu Fujii, Toshihisa Hamada, Takatoshi Shimauchi, Jun Asai, Yasuhiro Fujisawa, Hironobu Ihn, Norito Katoh
雑誌名: J Dermatol Sci. 2020 Mar;97(3):187-193. doi: 10.1016/j.jdermsci.2020.01.010. Epub 2020 Jan 25.
Abstract/Text BACKGROUND: The types of cutaneous lymphoma (CL) and their incidences can vary among geographic areas or ethnic groups.
OBJECTIVE: This study aimed to investigate the incidence of various CL types in Japan using epidemiological data from a nationwide registration system for CL.
METHODS: A questionnaire was sent to participating hospitals, all of which had been approved to conduct residency programs for board-certified dermatologists by the Japanese Dermatological Association. Data from patients newly diagnosed with CL were collected electronically.
RESULTS: Between 2012 and 2017, 2547 new patients with CL from the dermatological institutes were registered. In total, 2090 patients had primary CL and 453 had secondary CL. Those with primary CL included 1609 (77.0 %) patients with mature T- and natural killer (NK)-cell neoplasms, 442 (21.1 %) with B-cell neoplasms, and 39 (1.9 %) with blastic plasmacytoid dendritic cell neoplasms. Mycosis fungoides (MF) was the most common CL subtype in the present study (1003 patients, 48.0 %), and 72.4 % of MF patients had early-stage disease, similar to observations in previous studies on other cohorts. Primary cutaneous CD30+ T-cell lymphoproliferative disorders and adult T-cell leukemia/lymphoma were the second and third most common subtypes, respectively.
CONCLUSION: Compared to that in our previous cohort (2007-2011), the number of registered T- and NK-cell CL cases decreased, whereas that of B-cell CL cases increased from 44.8-73.7 patients/year. These results provide insight into CL trends within the Japanese population, which might contribute to a better understanding of the disease.

Copyright © 2020 Japanese Society for Investigative Dermatology. Published by Elsevier B.V. All rights reserved.
PMID 32033869  J Dermatol Sci. 2020 Mar;97(3):187-193. doi: 10.1016/j.・・・
著者: Shin-ya Suzuki, Kaoru Ito, Masaaki Ito, Kazuhiro Kawai
雑誌名: J Dermatol Sci. 2010 Jan;57(1):37-43. doi: 10.1016/j.jdermsci.2009.10.010.
Abstract/Text BACKGROUND: Survival analysis of a large series of patients with mycosis fungoides (MF) and Sézary syndrome (SS) has not been performed in Japan. Revision to the staging system for MF and SS was recently published.
OBJECTIVE: To determine the long-term prognosis of Japanese patients with MF and SS, to identify factors predictive of survival, and to evaluate the prognostic significance of the revised staging system.
METHODS: We performed a retrospective cohort study of 100 Japanese patients with MF and SS managed at the dermatology division of Niigata University Hospital between April 1, 1982 and March 31, 2006. We estimated survival according to the patient's clinical characteristics including the stages, and assessed their prognostic significance.
RESULTS: Survival rates of Japanese patients with MF and SS were similar to those shown in previous studies conducted in the United States and Europe. Prognosis of patients with skin tumor (stage IIB) and extracutaneous involvement (stage IV) was significantly worse than that of those with early-stage disease (stages IA-IIA), but erythrodermic MF patients without blood involvement (stage IIIA) showed excellent survival. Independent prognostic factors in multivariate analyses were higher age and the presence of skin tumor and extracutaneous disease.
CONCLUSION: Patient age and stage are the most important clinical prognostic factors in Japanese patients with MF and SS. The revised staging system is useful for predicting survival of the patients, but at least a subpopulation of stage IIIA patients may have a favorable prognosis.

Copyright 2009 Japanese Society for Investigative Dermatology. Published by Elsevier Ireland Ltd. All rights reserved.
PMID 19931424  J Dermatol Sci. 2010 Jan;57(1):37-43. doi: 10.1016/j.jd・・・
著者: Nita Sally Agar, Emma Wedgeworth, Siobhan Crichton, Tracey J Mitchell, Michael Cox, Silvia Ferreira, Alistair Robson, Eduardo Calonje, Catherine M Stefanato, Elizabeth Mary Wain, Bridget Wilkins, Paul A Fields, Alan Dean, Katherine Webb, Julia Scarisbrick, Stephen Morris, Sean J Whittaker
雑誌名: J Clin Oncol. 2010 Nov 1;28(31):4730-9. doi: 10.1200/JCO.2009.27.7665. Epub 2010 Sep 20.
Abstract/Text PURPOSE: We have analyzed the outcome of mycosis fungoides (MF) and Sézary syndrome (SS) patients using the recent International Society for Cutaneous Lymphomas (ISCL)/European Organisation for Research and Treatment of Cancer (EORTC) revised staging proposal.
PATIENTS AND METHODS: Overall survival (OS), disease-specific survival (DSS), and risk of disease progression (RDP) were calculated for a cohort of 1,502 patients using univariate and multivariate models.
RESULTS: The mean age at diagnosis was 54 years, and 71% of patients presented with early-stage disease. Disease progression occurred in 34%, and 26% of patients died due to MF/SS. A significant difference in survival and progression was noted for patients with early-stage disease having patches alone (T1a/T2a) compared with those having patches and plaques (T1b/T2b). Univariate analysis established that (1) advanced skin and overall clinical stage, increased age, male sex, increased lactate dehydrogenase (LDH), and large-cell transformation were associated with reduced survival and increased RDP; (2) hypopigmented MF, MF with lymphomatoid papulosis, and poikilodermatous MF were associated with improved survival and reduced RDP; and (3) folliculotropic MF was associated with an increased RDP. Multivariate analysis established that (1) advanced skin (T) stage, the presence in peripheral blood of the tumor clone without Sézary cells (B0b), increased LDH, and folliculotropic MF were independent predictors of poor survival and increased RDP; (2) large-cell transformation and tumor distribution were independent predictors of increased RDP only; and (3) N, M, and B stages; age; male sex; and poikilodermatous MF were only significant for survival.
CONCLUSION: This study has validated the recently proposed ISCL/EORTC staging system and identified new prognostic factors.

PMID 20855822  J Clin Oncol. 2010 Nov 1;28(31):4730-9. doi: 10.1200/JC・・・
著者: Julia J Scarisbrick, H Miles Prince, Maarten H Vermeer, Pietro Quaglino, Steven Horwitz, Pierluigi Porcu, Rudolf Stadler, Gary S Wood, Marie Beylot-Barry, Anne Pham-Ledard, Francine Foss, Michael Girardi, Martine Bagot, Laurence Michel, Maxime Battistella, Joan Guitart, Timothy M Kuzel, Maria Estela Martinez-Escala, Teresa Estrach, Evangelia Papadavid, Christina Antoniou, Dimitis Rigopoulos, Vassilki Nikolaou, Makoto Sugaya, Tomomitsu Miyagaki, Robert Gniadecki, José Antonio Sanches, Jade Cury-Martins, Denis Miyashiro, Octavio Servitje, Cristina Muniesa, Emilio Berti, Francesco Onida, Laura Corti, Emilia Hodak, Iris Amitay-Laish, Pablo L Ortiz-Romero, Jose L Rodríguez-Peralto, Robert Knobler, Stefanie Porkert, Wolfgang Bauer, Nicola Pimpinelli, Vieri Grandi, Richard Cowan, Alain Rook, Ellen Kim, Alessandro Pileri, Annalisa Patrizi, Ramon M Pujol, Henry Wong, Kelly Tyler, Rene Stranzenbach, Christiane Querfeld, Paolo Fava, Milena Maule, Rein Willemze, Felicity Evison, Stephen Morris, Robert Twigger, Rakhshandra Talpur, Jinah Kim, Grant Ognibene, Shufeng Li, Mahkam Tavallaee, Richard T Hoppe, Madeleine Duvic, Sean J Whittaker, Youn H Kim
雑誌名: J Clin Oncol. 2015 Nov 10;33(32):3766-73. doi: 10.1200/JCO.2015.61.7142. Epub 2015 Oct 5.
Abstract/Text PURPOSE: Advanced-stage mycosis fungoides (MF; stage IIB to IV) and Sézary syndrome (SS) are aggressive lymphomas with a median survival of 1 to 5 years. Clinical management is stage based; however, there is wide range of outcome within stages. Published prognostic studies in MF/SS have been single-center trials. Because of the rarity of MF/SS, only a large collaboration would power a study to identify independent prognostic markers.
PATIENTS AND METHODS: Literature review identified the following 10 candidate markers: stage, age, sex, cutaneous histologic features of folliculotropism, CD30 positivity, proliferation index, large-cell transformation, WBC/lymphocyte count, serum lactate dehydrogenase, and identical T-cell clone in blood and skin. Data were collected at specialist centers on patients diagnosed with advanced-stage MF/SS from 2007. Each parameter recorded at diagnosis was tested against overall survival (OS).
RESULTS: Staging data on 1,275 patients with advanced MF/SS from 29 international sites were included for survival analysis. The median OS was 63 months, with 2- and 5-year survival rates of 77% and 52%, respectively. The median OS for patients with stage IIB disease was 68 months, but patients diagnosed with stage III disease had slightly improved survival compared with patients with stage IIB, although patients diagnosed with stage IV disease had significantly worse survival (48 months for stage IVA and 33 months for stage IVB). Of the 10 variables tested, four (stage IV, age > 60 years, large-cell transformation, and increased lactate dehydrogenase) were independent prognostic markers for a worse survival. Combining these four factors in a prognostic index model identified the following three risk groups across stages with significantly different 5-year survival rates: low risk (68%), intermediate risk (44%), and high risk (28%).
CONCLUSION: To our knowledge, this study includes the largest cohort of patients with advanced-stage MF/SS and identifies markers with independent prognostic value, which, used together in a prognostic index, may be useful to stratify advanced-stage patients.

© 2015 by American Society of Clinical Oncology.
PMID 26438120  J Clin Oncol. 2015 Nov 10;33(32):3766-73. doi: 10.1200/・・・
著者: Rein Willemze, Lorenzo Cerroni, Werner Kempf, Emilio Berti, Fabio Facchetti, Steven H Swerdlow, Elaine S Jaffe
雑誌名: Blood. 2019 Apr 18;133(16):1703-1714. doi: 10.1182/blood-2018-11-881268. Epub 2019 Jan 11.
Abstract/Text Primary cutaneous lymphomas are a heterogeneous group of T- and B-cell lymphomas that present in the skin with no evidence of extracutaneous disease at the time of diagnosis. The 2005 World Health Organization-European Organization for Research and Treatment of Cancer (WHO-EORTC) consensus classification has served as a golden standard for the diagnosis and classification of these conditions. In September 2018, an updated version of the WHO-EORTC was published in the fourth edition of the WHO Classification of Skin Tumours Blue Book. In this classification, primary cutaneous acral CD8+ T-cell lymphoma and Epstein-Barr virus positive (EBV+) mucocutaneous ulcer are included as new provisional entities, and a new section on cutaneous forms of chronic active EBV disease has been added. The term "primary cutaneous CD4+ small/medium T-cell lymphoma" was modified to "primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder" because of its indolent clinical behavior and uncertain malignant potential. Modifications have also been made in the sections on lymphomatoid papulosis, increasing the spectrum of histologic and genetic types, and primary cutaneous marginal zone lymphomas recognizing 2 different subtypes. Herein, the characteristic features of these new and modified entities as well as the results of recent molecular studies with diagnostic, prognostic, and/or therapeutic significance for the different types of primary cutaneous lymphomas are reviewed. An update of the frequency and survival of the different types of primary cutaneous lymphomas is provided.

© 2019 by The American Society of Hematology.
PMID 30635287  Blood. 2019 Apr 18;133(16):1703-1714. doi: 10.1182/bloo・・・
著者: Elise A Olsen, Sean Whittaker, Youn H Kim, Madeleine Duvic, H Miles Prince, Stuart R Lessin, Gary S Wood, Rein Willemze, Marie-France Demierre, Nicola Pimpinelli, Maria Grazia Bernengo, Pablo L Ortiz-Romero, Martine Bagot, Teresa Estrach, Joan Guitart, Robert Knobler, José Antonio Sanches, Keiji Iwatsuki, Makoto Sugaya, Reinhard Dummer, Mark Pittelkow, Richard Hoppe, Sareeta Parker, Larisa Geskin, Lauren Pinter-Brown, Michael Girardi, Günter Burg, Annamari Ranki, Maartan Vermeer, Steven Horwitz, Peter Heald, Steve Rosen, Lorenzo Cerroni, Brigette Dreno, Eric C Vonderheid, International Society for Cutaneous Lymphomas, United States Cutaneous Lymphoma Consortium, Cutaneous Lymphoma Task Force of the European Organisation for Research and Treatment of Cancer
雑誌名: J Clin Oncol. 2011 Jun 20;29(18):2598-607. doi: 10.1200/JCO.2010.32.0630. Epub 2011 May 16.
Abstract/Text Mycosis fungoides (MF) and Sézary syndrome (SS), the major forms of cutaneous T-cell lymphoma, have unique characteristics that distinguish them from other types of non-Hodgkin's lymphomas. Clinical trials in MF/SS have suffered from a lack of standardization in evaluation, staging, assessment, end points, and response criteria. Recently defined criteria for the diagnosis of early MF, guidelines for initial evaluation, and revised staging and classification criteria for MF and SS now offer the potential for uniform staging of patients enrolled in clinical trials for MF/SS. This article presents consensus recommendations for the general conduct of clinical trials of patients with MF/SS as well as methods for standardized assessment of potential disease manifestations in skin, lymph nodes, blood, and visceral organs, and definition of end points and response criteria. These guidelines should facilitate collaboration among investigators and collation of data from sponsor-generated or investigator-initiated clinical trials involving patients with MF or SS.

PMID 21576639  J Clin Oncol. 2011 Jun 20;29(18):2598-607. doi: 10.1200・・・
著者: Yu Sawada, Ryosuke Hino, Kayo Hama, Shun Ohmori, Haruna Fueki, Shigenori Yamada, Shoko Fukamachi, Makiko Tajiri, Rieko Kubo, Manabu Yoshioka, Daiki Nakashima, Kazunari Sugita, Ryutaro Yoshiki, Takatoshi Shimauchi, Tomoko Mori, Kunio Izu, Miwa Kobayashi, Motonobu Nakamura, Yoshiki Tokura
雑誌名: Blood. 2011 Apr 14;117(15):3961-7. doi: 10.1182/blood-2010-11-316794. Epub 2011 Feb 16.
Abstract/Text Cutaneous involvement is seen in ~ 50% of adult T-cell leukemia/lymphoma (ATLL) patients. We investigated the association between skin eruption type and prognosis in 119 ATLL patients. ATLL eruptions were categorized into patch (6.7%), plaque (26.9%), multipapular (19.3%), nodulotumoral (38.7%), erythrodermic (4.2%), and purpuric (4.2%) types. When the T stage of the tumor-node-metastasis-blood (TNMB) classification of mycosis fungoides/Sézary syndrome was applied to ATLL staging, 16.0% were T1, 17.7% T2, 38.7% T3, and 4.2% T4, and the remaining 23.5% were of the multipapular and purpuric types. For the patch type, the mean survival time (median survival time could not be estimated) was 188.4 months. The median survival times (in months) for the remaining types were as follows: plaque, 114.9; multipapular, 17.3; nodulotumoral, 17.3; erythrodermic, 3.0; and purpuric, 4.4. Kaplan-Meier curves of overall survival showed that the erythrodermic type had the poorest prognosis, followed by the nodulotumoral and multipapular types. The patch and plaque types were associated with better survival rates. Multivariate analysis demonstrated that the hazard ratios of the erythrodermic and nodulotumoral types were significantly higher than that of the patch type, and that the eruption type is an independent prognostic factor for ATLL. The overall survival was worse as the T stage became more advanced: the multipapular type and T2 were comparable, and the purpuric type had a significantly poorer prognosis than T1.

PMID 21325600  Blood. 2011 Apr 14;117(15):3961-7. doi: 10.1182/blood-2・・・
著者: Rein Willemze, Patty M Jansen, Lorenzo Cerroni, Emilio Berti, Marco Santucci, Chalid Assaf, Marijke R Canninga-van Dijk, Agnes Carlotti, Marie-Louise Geerts, Sonja Hahtola, Michael Hummel, Leila Jeskanen, Werner Kempf, Cesare Massone, Pablo L Ortiz-Romero, Marco Paulli, Tony Petrella, Annamari Ranki, José L Rodriguez Peralto, Alistair Robson, Nancy J Senff, Maarten H Vermeer, Janine Wechsler, Sean Whittaker, Chris J L M Meijer, EORTC Cutaneous Lymphoma Group
雑誌名: Blood. 2008 Jan 15;111(2):838-45. doi: 10.1182/blood-2007-04-087288. Epub 2007 Oct 12.
Abstract/Text In the WHO classification, subcutaneous panniculitis-like T-cell lymphoma (SPTL) is defined as a distinct type of T-cell lymphoma with an aggressive clinical behavior. Recent studies suggest that distinction should be made between SPTL with an alpha/beta T-cell phenotype (SPTL-AB) and SPTL with a gammadelta T-cell phenotype (SPTL-GD), but studies are limited. To better define their clinicopathologic features, immunophenotype, treatment, and survival, 63 SPTL-ABs and 20 SPTL-GDs were studied at a workshop of the EORTC Cutaneous Lymphoma Group. SPTL-ABs were generally confined to the subcutis, had a CD4-, CD8+, CD56-, betaF1+ phenotype, were uncommonly associated with a hemophagocytic syndrome (HPS; 17%), and had a favorable prognosis (5-year overall survival [OS]: 82%). SPTL-AB patients without HPS had a significantly better survival than patients with HPS (5-year OS: 91% vs 46%; P<.001). SPTL-GDs often showed (epi)dermal involvement and/or ulceration, a CD4-, CD8-, CD56+/-, betaF1- T-cell phenotype, and poor prognosis (5-year OS: 11%), irrespective of the presence of HPS or type of treatment. These results indicate that SPTL-AB and SPTL-GD are distinct entities, and justify that the term SPTL should further be used only for SPTL-AB. SPTL-ABs without associated HPS have an excellent prognosis, and multiagent chemotherapy as first choice of treatment should be questioned.

PMID 17934071  Blood. 2008 Jan 15;111(2):838-45. doi: 10.1182/blood-20・・・
著者: H S Zackheim, M Kashani-Sabet, S Amin
雑誌名: Arch Dermatol. 1998 Aug;134(8):949-54.
Abstract/Text OBJECTIVE: To determine the effectiveness of topical corticosteroids in the management of mycosis fungoides.
DESIGN: Prospective study.
SETTING: Academic referral center, Veterans Affairs Medical Center, and private practice.
PATIENTS: Seventy-nine patients with patch or plaque stage of mycosis fungoides. Fifty-one were stage T1 (less than 10% of skin involved) and 28 were stage T2 (10% or more of skin involved). Seventy-five had patch-stage and 4 had plaque-stage disease as determined by histological examination.
INTERVENTION: Patients were treated with topical class I to III corticosteroids. Of the stage T1 patients, all used class I corticosteroids, and 4 (8%) also used class II or III corticosteroids. Of the stage T2 patients, 19 (68%) used class I and 12 (43%) used class II or III compounds. Some patients used more than 1 class of corticosteroid. Applications were almost always twice daily. Three stage T1 and 2 stage T2 patients used plastic film occlusion. Baseline and monthly morning serum cortisol levels were obtained during treatment.
MAIN OUTCOME MEASURES: Response to treatment and side effects.
RESULTS: The median follow-up period was 9 months. Thirty-two (63%) of stage T1 patients achieved complete remission and 16 (31%) achieved partial remission, for a total response rate of 48 (94%). The comparable figures for stage T2 patients were 7 (25%), 16 (57%), and 23 (82%), respectively. Responses were determined by clinical examination. Thirty-nine patients achieved clinical clearing. In 7 of these, posttreatment biopsy specimens were obtained, and all showed histological clearing. Reversible depression of serum cortisol levels occurred in 10 (13%). Minor skin irritation occurred in 2 patients and localized, reversible skin atrophy in 1.
CONCLUSION: Topical corticosteroids, especially class I compounds, are an effective treatment for patch-stage mycosis fungoides.

PMID 9722724  Arch Dermatol. 1998 Aug;134(8):949-54.
著者: F Pavlotsky, A Barzilai, R Kasem, D Shpiro, H Trau
雑誌名: J Eur Acad Dermatol Venereol. 2006 May;20(5):565-72. doi: 10.1111/j.1468-3083.2006.01557.x.
Abstract/Text BACKGROUND: Several options for treatment of early mycosis fungoides (MF) offer similar success rates. Previous small studies have shown UVB to be at least as effective as PUVA.
OBJECTIVE: To summarize our experience with UVB treatment of early MF.
METHODS: A retrospective analysis of early-stage MF patients treated by narrow band (NB) or broad band (BB) UVB in our institution between 1996 and 2002. Most patients achieving complete response (CR) were put on maintenance until natural sun exposure was possible and followed up every 3-6 months. The results were compared to those previously reported regarding PUVA.
RESULTS: Sixty-eight and 43 patients were treated by NB and BB UVB, respectively. Eighty-six per cent (84 and 89% in NB and BB UVB groups, respectively) of IA and 71% (78 and 44% in NB and BB UVB groups, respectively) of IB patients achieved CR within a mean of 12.8 and 10.6 weeks, respectively. When maintenance was stopped, 65 and 30% had not relapsed after an average follow up of 27 and 222 weeks, respectively. Non-relapse rate was 33 and 48% for those having had vs. those not having had maintenance, respectively.
CONCLUSIONS: Our results are comparable to all previously reported for skin-targeted treatments, including PUVA and, to our belief, reflect the nature of early MF, in which CR can probably be achieved in most of the patients. Among the responding patients there is no relapse during prolonged follow-up in about one third of the cases. Thus, we believe treatment should be stopped completely following first CR induction and maintenance treatment should be considered for relapsing patients only. Both broad and narrow UVB options are good and future choices should be made on the basis of short- and long-term side-effects.

PMID 16684285  J Eur Acad Dermatol Venereol. 2006 May;20(5):565-72. do・・・
著者: Gonca Boztepe, Sedef Sahin, Meltem Ayhan, Gul Erkin, Fikret Kilemen
雑誌名: J Am Acad Dermatol. 2005 Aug;53(2):242-6. doi: 10.1016/j.jaad.2005.03.012.
Abstract/Text BACKGROUND AND PURPOSE: Narrowband ultraviolet B (UVB) phototherapy for early-stage mycosis fungoides (MF) has been found to be beneficial in some reports. Although rapid recurrences after discontinuation of therapy appear to interfere with its efficacy, optimal maintenance schedules for prolonged relapse-free intervals are not discussed in the literature. The purpose of this study was to review our experience with narrowband UVB in patients with MF.
PATIENTS AND METHODS: All available data that belong to 14 patients (10 male, 4 female; age range, 28-74 years) with histologically proven MF, at disease stages IA-IB (n = 10) and IIA (n = 4) who received narrowband UVB were retrospectively evaluated.
RESULTS: Complete response (CR) was achieved in 11 of 14 cases (78%) after a mean of 25 treatments. Ten of 11 patients were followed up for a median of 22 months (range, 7-43 months) after CR; one patient was lost to follow-up immediately after CR. Eight patients completed the recommended maintenance narrowband UVB therapy protocol. The median duration of maintenance was 18 months (range, 12-30 months). No patient had relapse during maintenance. Mean relapse-free duration was 26.0 +/- 9.9 months.
LIMITATIONS: The number of patients in the study group was relatively few.
CONCLUSION: This study provides evidence that narrowband UVB might be an efficient treatment option for MF patients at stages IA and IB, as well as at stage IIA. Results suggest that using maintenance phototherapy after CR is a logical approach, which may prolong the duration of remission in MF.

PMID 16021117  J Am Acad Dermatol. 2005 Aug;53(2):242-6. doi: 10.1016/・・・
著者: Christiane Querfeld, Steven T Rosen, Timothy M Kuzel, Katharine A Kirby, Henry H Roenigk, Bettina M Prinz, Joan Guitart
雑誌名: Arch Dermatol. 2005 Mar;141(3):305-11. doi: 10.1001/archderm.141.3.305.
Abstract/Text OBJECTIVES: To evaluate long-term outcomes, impact of maintenance therapy and potential curability of patients with mycosis fungoides (MF) treated with psoralen plus UV-A (PUVA) monotherapy.
DESIGN: Single-center retrospective cohort analysis.
SETTING: Academic referral center for cutaneous lymphoma.
PATIENTS: A total of 66 of 104 patients with clinical stages IA to IIA MF who achieved complete remission (CR) after PUVA monotherapy between 1979 and 1995.
MAIN OUTCOME MEASURES: Kaplan-Meier actuarial survival and disease-free survival curves were compared between stage IA and IB/IIA cases. Patients were stratified into relapse and nonrelapse groups based on whether their MF relapsed during study follow-up. Baseline characteristics and treatment were compared between these groups.
RESULTS: Median follow-up time was 94 months (range, 5-242 months). Thirty-three patients maintained CR for 84 months (range, 5-238 months), and 33 patients experienced relapse with a median disease-free interval of 39 months (range, 2-127 months). There was no significant difference in baseline characteristics between patients in the nonrelapse and relapse groups. Those in the nonrelapse group received a higher cumulative dosage to CR (P = .03) and required longer treatment periods to achieve CR (P = .03). Disease-free survival rates at 5 and 10 years for all patients with stage IA were 56% and 30%, respectively, and for stage IB/IIA, 74% and 50%. Actuarial survival rates at 5, 10, and 15 years were 94%, 82%, and 82%, respectively, in patients with stage IA, and 80%, 69%, and 58% in patients with stage IB/IIA. The overall survival rate for the nonrelapse and relapse groups did not show any statistical difference. One third of the patients developed signs of chronic photodamage and secondary cutaneous malignancies.
CONCLUSIONS: Psoralen UV-A is an effective treatment for MF, inducing long-term remissions and perhaps in some cases disease "cure." Thirty percent to 50% of patients remain disease free for 10 years, but late relapses occur. Long-term survival is not affected by relapse status, and the risk of photodamage needs to be measured against the possible benefit of greater disease elimination.

PMID 15781671  Arch Dermatol. 2005 Mar;141(3):305-11. doi: 10.1001/arc・・・
著者: J J Herrmann, H H Roenigk, A Hurria, T M Kuzel, E Samuelson, A W Rademaker, S T Rosen
雑誌名: J Am Acad Dermatol. 1995 Aug;33(2 Pt 1):234-42.
Abstract/Text BACKGROUND: Mycosis fungoides (MF) is a non-Hodgkin's T-cell lymphoma of the skin that often begins as limited patches and plaques with slow progression to systemic involvement. No studies have been published comparing photochemotherapy (PUVA) with other topical therapies in the treatment of early-stage disease.
OBJECTIVE: The purpose of the study was to examine our long-term experience using PUVA to treat early-stage MF and to compare its effectiveness and side-effect profile with other previously reported topical therapies.
METHODS: Eighty-two patients with MF (83% stage IA or IB) were treated with PUVA. Clinical and histologic features were observed for a period from 2 months to 15 years (median, 43 months).
RESULTS: A response was noted in 78 patients (95%) with complete clinical and histologic clearing in 53 patients (65%) for all stages. The mean duration of total complete response to PUVA for all stages was 43 months (3.6 years). The mean survival of our study group for all stages was 8.5 years. Signs of chronic actinic skin damage were found in 10% of patients, including three patients with basal cell carcinomas and three patients with squamous cell carcinomas. In a nonrandomized comparison with previously reported data for other topical therapies, the efficacy and side-effect profile of PUVA compared favorably.
CONCLUSION: PUVA is an effective and safe therapy for MF with prolonged disease-free remissions being achieved. Patients with stage I and II MF respond best to PUVA. Palliative therapy with PUVA is useful in more advanced cases of MF.

PMID 7622650  J Am Acad Dermatol. 1995 Aug;33(2 Pt 1):234-42.
著者: Florian Weber, Matthias Schmuth, Norbert Sepp, Peter Fritsch
雑誌名: Acta Derm Venereol. 2005;85(4):329-32. doi: 10.1080/00015550510032814.
Abstract/Text PUVA therapy is widely used for early stage mycosis fungoides. While the efficacy of PUVA with oral 8-methoxypsoralen (8-MOP) is well documented, the use of its topical variation, bath-water PUVA therapy with 8-MOP has not been studied. The purpose of this study was to assess the effect of 8-MOP bath-water PUVA therapy in adult patients with early stage mycosis fungoides. We retrospectively evaluated the outcomes of bath-water delivery of 8-MOP (1 mg l(-1)) in 16 patients with early stage mycosis fungoides. In all patients complete response was achieved after a mean duration of 63 days requiring 29 treatments and a mean cumulative UVA dose of 33 J cm(-2). The time to relapse after complete clinical clearance was 45.6 (+/-9.2) weeks. In comparison, oral PUVA therapy with 8-MOP resulted in complete response after 64.5 days (25.8 treatments) with a mean relapse-free period of 30 (+/-3.5) weeks. We conclude that bath-water PUVA therapy with 8-MOP is a valuable photo-therapeutic alternative, which should be considered for patients in whom systemic psoralen cannot be used.

PMID 16191854  Acta Derm Venereol. 2005;85(4):329-32. doi: 10.1080/000・・・
著者: B Micaily, C Miyamoto, G Kantor, S Lessin, A Rook, L Brady, R Goodman, E C Vonderheid
雑誌名: Int J Radiat Oncol Biol Phys. 1998 Sep 1;42(2):361-4.
Abstract/Text PURPOSE: To evaluate the treatment outcome and natural history of patients with the diagnosis of unilesional mycosis fungoides, treated according to a prospective radiotherapy protocol in our institution since July 1975.
METHODS AND MATERIALS: A total of 325 patients with the diagnosis of mycosis fungoides have been referred to the Department of Radiation Oncology at Allegheny University of Health Sciences from July 1975 through September 1996. Of these, 18 patients (5%) were classified as having unilesional mycosis fungoides and were irradiated with a curative intent using local electron fields. One patient received 22 Gy; 1 patient received 40 Gy, and the rest of the patients 30.6 Gy. Daily fractions ranged from 1.8 to 2.0 Gy. Treatments prior to radiation consisted of topical steroids and/or antifungal creams in the majority of patients, with temporary partial responses. One patient had received 2 years of topical mechlorethamine (HN2) and another patient had received topical carmustine solution (BCNU) without response prior to irradiation.
RESULTS: The responses were measured clinically; posttreatment skin biopsy was not performed routinely unless there was clinical evidence of disease persistence. Complete response rate was 100%; all treated lesions cleared completely within 4 to 8 weeks after the completion of radiation. With a median follow-up of 43 months (range 12 to 240 months), 2 relapses have occurred, 2 and 71 months after the completion of radiation. Both relapses were confined to the skin and were remote from the original site. Both relapses responded to topical application of HN2. There have been no recurrences in the irradiated field nor systemic dissemination. No long-term side effects were found related to treatment, and all the patients are currently alive and without evidence of disease. Actuarial relapse-free and overall survival at 10 years are, respectively, 86.2% and 100%.
CONCLUSION: Unilesional mycosis fungoides has a long natural history, is possibly the earliest manifestation of a malignant process, and local treatments, including local radiotherapy, result in long-term disease-free intervals and, possibly, cure. Total skin electron beam radiotherapy is not indicated for this disease entity.

PMID 9788416  Int J Radiat Oncol Biol Phys. 1998 Sep 1;42(2):361-4.
著者: Glenn W Jones, Barry M Kacinski, Lynn D Wilson, Rein Willemze, Margaret Spittle, Gerda Hohenberg, Leonore Handl-Zeller, Franz Trautinger, Robert Knobler
雑誌名: J Am Acad Dermatol. 2002 Sep;47(3):364-70.
Abstract/Text Radiotherapy has been successfully implemented in the treatment of mycosis fungoides (MF) for almost a century. With the development of the modern linear accelerator, it has become possible to treat extended areas of the skin with accelerated electrons. Total skin electron beam radiation (TSEB) has been in use for several decades, and a number of technical modifications have been made with the goals of optimizing dose distribution and improving clinical outcome. Emerging evidence from recent studies suggests an association between TSEB techniques and efficacy in the treatment of MF. Based on this evidence, the European Organization for Research and Treatment of Cancer Cutaneous Lymphoma Project Group, in association with experts from radiotherapy centers in North America, has reached a consensus on acceptable methods and clinical indications for TSEB in the treatment of MF. The aims of this report are to enhance accessibility of this highly efficacious treatment modality to patients with MF and to provide a point of reference for further clinical research.

PMID 12196745  J Am Acad Dermatol. 2002 Sep;47(3):364-70.
著者: P A Quiros, B M Kacinski, L D Wilson
雑誌名: Cancer. 1996 May 1;77(9):1912-7. doi: 10.1002/(SICI)1097-0142(19960501)77:9<1912::AID-CNCR23>3.0.CO;2-1.
Abstract/Text BACKGROUND: The goal of this study was to define the prognostic significance of the extent of skin involvement (ESI) with respect to disease free survival (DFS) and overall survival (OS) of patients with T3 cutaneous T-cell lymphoma (CTCL) after total skin electron beam therapy (TSEBT).
METHODS: Between 1974 and 1993, TSEBT (36 Gray [Gy], 1 Gy/day for 9 weeks, 6 MeV electrons) was administered to a total of 213 patients. Forty-six of the 213 patients were classified as having T3 CTCL based on the presence of tumor nodules on the skin at diagnosis. Patient records were evaluated retrospectively, and the percentage of total skin surface involved was calculated. Patients were analyzed with respect to response to therapy, disease free and overall survival. The median follow-up was 37.5 months (range, 1.6-93 months).
RESULTS: Thirty-six of 46 patients achieved complete clinical response (CCR) by the completion of TSEBT. DFS was 12% at 36 months with approximately 28% OS. When stratified by extent of skin involvement, 100% of patients with 9% or less ESI were disease free at 18 months compared with patients with 10% or greater ESI, all of whom had relapsed by 18 months (78% achieved CCR). Fifty percent of those with 9% or less ESI remained disease free at 36 months; median DFS and OS were not reached at 63 and 65 months, respectively. The median DFS and OS for the 10% or greater ESI group were 4 and 24 months, respectively. These differences were statistically significant (P < or = 0.005). Toxicity of therapy was minimal.
CONCLUSIONS: The extent of skin involvement of patients with T3 CTCL is a prognostic indicator of disease free and overall survival for those who have been treated definitively with TSEBT.

PMID 8646693  Cancer. 1996 May 1;77(9):1912-7. doi: 10.1002/(SICI)109・・・
著者: G W Jones, D Rosenthal, L D Wilson
雑誌名: Cancer. 1999 May 1;85(9):1985-95.
Abstract/Text BACKGROUND: There is limited published evidence regarding the efficacy of total skin electron beam radiation for patients with the diffuse erythrodermic form of mycosis fungoides.
METHODS: Forty-five patients with erythrodermic mycosis fungoides were managed at McMaster University in Hamilton, Ontario, Canada (n=34), and at Yale University (n=11) between 1970 and 1996. All received radiation without neoadjuvant, concomitant, or adjuvant therapies. The median age was 67 years (range, 42-84 years). The male-to-female ratio was 2.2. Fifteen received radiation for the treatment of newly diagnosed disease. There were 28 with Stage III (T4 N0-1 M0), 13 with Stage IVA (T4 N2-3 M0), and 4 with Stage IVB (T4 N0-3 M1) disease, and 21 had blood involvement. The median radiation dose was 32 gray (Gy) (range, 4.8-40 Gy). The median treatment time was 21 days (range, 3-125 days). A technically more intense method of radiation (32-40 Gy and 4-6 MeV electrons) was administered to 23 patients.
RESULTS: All patients responded. The rate of complete cutaneous remission was 60%, with 26% remaining progression free at 5 years. Remission was associated with more intense radiation (P=0.014 in multivariate analysis with adjustment for blood and staging information). With the more intense radiation, 74% attained remission, with 36% remaining progression free at 5 years. For 8 patients with Stage III disease without blood involvement, all entered remission, with 69% remaining progression free at 5 years. Twenty of 30 deaths were related to mycosis fungoides. The median overall survival was 3.4 years, with a 10-year estimate of 28%. The median cause specific survival was 5 years, with a 10-year estimate of 43%. Both overall and cause specific survival were associated with an absence of blood involvement (both P<0.03 in multivariate analysis). Age was not a significant factor. Toxicities of radiation were acceptable when radiation was administered over 6-9 weeks at 5 fractions per week.
CONCLUSIONS: Total skin radiation is an efficient monotherapy for patients with erythrodermic mycosis fungoides. With more intense radiation, the rate of cutaneous remission is 74%, and 27% remain progression free at 10 years. Radiation may be most efficacious in Stage III, with no blood involvement. When there is blood, lymph node, or visceral involvement, combined modality therapies should be explored.

PMID 10223240  Cancer. 1999 May 1;85(9):1985-95.
著者: R Stadler, H G Otte, T Luger, B M Henz, P Kühl, T Zwingers, W Sterry
雑誌名: Blood. 1998 Nov 15;92(10):3578-81.
Abstract/Text Cutaneous T-cell lymphoma (CTCL) constitutes a malignant proliferative disease involving mostly CD4(+) T cells arising in the skin. Because of the lack of curative treatment options, interferons (IFN) have been introduced into the therapy of CTCL. Although effective even in advanced disease, response rates were about 50% and the duration of response was short. To improve the results of interferon monotherapy, combinations of IFN with oral photochemotherapy (PUVA) or retinoids were investigated in nonrandomized trials showing higher response rates. We have therefore conducted this prospective randomized multicenter trial to compare these two combination therapies, ie, IFN plus PUVA and IFN plus acitretin. IFN -2a was administered at 9 MU three times weekly subcutaneously in both groups, with lower increasing doses during the first week. Photochemotherapy was applied after oral intake of 8-methoxypsoralen (0.6 mg/kg body weight) 5x weekly during the first 4 weeks, 3x weekly from weeks 5 through 23, and 2x weekly from weeks 24 through 48, with escalating doses beginning with 0.25 J/cm2. Twenty-five milligrams of acitretin was administered daily during the first week, and 50 mg was administered from weeks 2 through 48. Of 98 patients randomized in this study, 82 stage I and II patients were evaluable: 40 in the IFN+PUVA group and 42 in the IFN+acitretin group. With 70% complete remissions in the IFN+PUVA group, this treatment was significantly superior to the IFN+acitretin group with only 38.1% complete remissions. Time to response was significantly shorter in the IFN+PUVA group, with 18.6 weeks compared with 21.8 weeks in the IFN+acitretin group. Side effects were mostly mild to moderate and did not differ significantly in both treatment groups. However, there were more adverse events leading to study discontinuation in the IFN+acitretin group. Based on these findings, we conclude that IFN plus oral photochemotherapy is superior to IFN plus acitretin, inducing more complete remissions in patients with CTCL stages I and II.

PMID 9808550  Blood. 1998 Nov 15;92(10):3578-81.
著者: L Molin, K Thomsen, G Volden, A Aronsson, H Hammar, L Hellbe, G L Wantzin, G Roupe
雑誌名: Acta Derm Venereol. 1987;67(3):232-6.
Abstract/Text Thirty-nine patients with mycosis fungoides in various stages or Sézary syndrome were treated with isotretinoin and 29 with etretinate as single drug therapy. Complete remission within 2 months was obtained with isotretinoin in 8 cases (21%) and partial remission in another 15 cases (38%). Etretinate induced complete remission in 5 cases (21%) and partial remission in 11 (46%). Only 1 case with Sézary syndrome went into partial remission. The first sign of remission occurred in 2 to 4 weeks. During continued treatment remissions could not always be maintained. Isotretinoin and etretinate were considered to be of equal potency in the treatment of mycosis fungoides.

PMID 2442936  Acta Derm Venereol. 1987;67(3):232-6.
著者: M Duvic, A G Martin, Y Kim, E Olsen, G S Wood, C A Crowley, R C Yocum, Worldwide Bexarotene Study Group
雑誌名: Arch Dermatol. 2001 May;137(5):581-93.
Abstract/Text OBJECTIVES: To determine the safety and efficacy of oral bexarotene (Targretin capsules; Ligand Pharmaceuticals Incorporated, San Diego, Calif).
DESIGN: The effects of 2 randomized doses of 6.5 mg/m(2) per day (with crossover for progression) vs 650 mg/m(2) per day (later modified to 300 mg/m(2) per day) were evaluated in an open-label, multicenter, phase 2 and 3 study conducted between February 1997 and November 1998.
SETTING: Eighteen international cutaneous T-cell lymphoma clinics at academic referral centers.
PATIENTS: Fifty-eight patients with biopsy-proven stage IA through IIA cutaneous T-cell lymphoma that was refractory to (or patients were intolerant of) treatment or had reached at least a 6-month response plateau under at least 2 forms of prior therapy (median of 3.5 prior therapies).
INTERVENTION: Bexarotene (Targretin capsules) administered once daily with meal for 16 weeks or longer.
MAIN OUTCOME MEASURES: Primary end point classification of overall response rate of complete and partial remissions determined by either the Physician's Global Assessment of Clinical Condition or the objective Composite Assessment of Index Lesion Severity. Body surface area, time to response, duration of disease control, time to disease progression, individual index lesion signs and symptoms, and quality of life parameters were secondary outcomes.
RESULTS: Responses (> or = 50% improvement) were seen in 3 (20%) of 15 patients with an initial dose at 6.5 mg/m(2) per day (95% confidence interval [CI], 0%-40%), 15 (54%) of 28 patients at 300 mg/m(2) per day (95% CI, 35%-72%), and 10 (67%) of 15 patients at above 300 mg/m(2) per day (95% CI, 43%-91%). The rate of progressive disease was 47%, 21%, and 13% at the same dose levels, respectively. Eight (73%) of 11 patients crossing over from 6.5 mg/m(2) per day to higher doses subsequently responded. The median duration of response from start of therapy could not be estimated for the 15 patients at 300 mg/m(2) per day owing to low relapse rates in 2 patients (13%); at higher doses it was 516 days. The following drug-related adverse effects were reversible and treatable: hypertriglyceridemia (46 patients [79%]), hypercholesterolemia (28 patients [48%]), headache (27 patients [47%]), central hypothyroidism (23 patients [40%]), asthenia (21 patients [36%]), and leukopenia (16 patients [28%]). No cases of drug-related neutropenic fever, sepsis, or death occurred. Pancreatitis occurred in 3 patients with triglyceride levels higher than 14.69 mmol/L (1300 mg/dL), all of whom were taking 300 mg/m(2) or more of oral bexarotene per day.
CONCLUSIONS: Bexarotene (Targretin capsules) (the first retinoid X receptor-selective rexinoid) was well tolerated and effective as an oral treatment for 15 (54%) of 28 patients with refractory or persistent early-stage cutaneous T-cell lymphoma at doses of 300 mg/m(2) per day. Hypertriglyceridemia and hypothyroidism require monitoring but are reversible and manageable with concomitant medication.

PMID 11346336  Arch Dermatol. 2001 May;137(5):581-93.
著者: M Duvic, K Hymes, P Heald, D Breneman, A G Martin, P Myskowski, C Crowley, R C Yocum, Bexarotene Worldwide Study Group
雑誌名: J Clin Oncol. 2001 May 1;19(9):2456-71.
Abstract/Text PURPOSE: Cutaneous T-cell lymphomas (CTCL) are malignancies of T cells appearing as skin lesions and are responsive to retinoid therapy. Safety and efficacy of a novel RXR-selective retinoid (rexinoid) bexarotene (Targretin, LGD1069; Ligand Pharmaceuticals Inc, San Diego, CA) was evaluated as a single-agent oral therapy administered once daily in an open-label study in patients with refractory advanced-stage CTCL.
PATIENTS AND METHODS: Ninety-four patients with biopsy-confirmed CTCL in advanced stages (IIB-IVB) were enrolled at 26 centers. Fifty-six patients received an initial dose of 300 mg/m2/d oral bexarotene and 38 started at more than 300 mg/m2/d.
RESULTS: Clinical complete and partial responses were reported by Primary End point Classification for the study in 45% (25 of 56) of patients enrolled at 300 mg/m2/d dosing. At more than 300 mg/m2/d, 55% (21 of 38) of patients responded, including 13% (five of 38) clinical complete. For the 300 mg/m2/d initial dose group, the rate of relapse after response was 36% and the projected median duration of response was 299 days. Improvements were also seen in overall body-surface area involvement, median index lesion surface area, adenopathy, cutaneous tumors, pruritus, and CTCL-specific quality of life. The most frequent drug-related adverse events included hypertriglyceridemia (associated rarely with pancreatitis), hypercholesterolemia, hypothyroidism, and headache.
CONCLUSION: Bexarotene is the first in a novel class of pharmacologic agents, the RXR-selective retinoids, or rexinoids. Bexarotene is orally administered, safe, and generally well tolerated with reversible side effects, and is effective for the treatment of advanced, refractory CTCL.

PMID 11331325  J Clin Oncol. 2001 May 1;19(9):2456-71.
著者: P N Grozea, S E Jones, E M McKelvey, C A Coltman, R Fisher, C L Haskins
雑誌名: Cancer Treat Rep. 1979 Apr;63(4):647-53.
Abstract/Text Between 1972 and 1977, the Southwest Oncology Group studied the following three chemotherapy programs for the treatment of patients with advanced forms of mycosis fungoides: (a) cyclophosphamide, adriamycin, vincristine, and prednisone (CHOP) (seven patients); (b) adriamycin, vincristine, and prednisone (HOP) (five patients); and (c) cyclophosphamide, vincristine, prednisone, and bleomycin (COP plus bleomycin) (12 patients). Among the 24 evaluable patients there was an overall objective response rate of 95% with seven (29%) achieving a complete remission. With the adriamycin-containing chemotherapy, five (42%) of 12 patients achieved a complete remission compared to two (17%) of 12 patients treated with COP plus bleomycin. The median duration of remission (partial plus complete) was longer with the COP plus bleomycin combination (median, 47 weeks) than with the adriamycin-containing combinations (median, 22 weeks; P = 0.03). The median survival for all 24 evaluable patients was 95 weeks and was similar regardless of remission-induction therapy. In summary, combination chemotherapy proved to be effective palliative therapy for advanced mycosis fungoides.

PMID 87277  Cancer Treat Rep. 1979 Apr;63(4):647-53.
著者: M T Fierro, G C Doveil, P Quaglino, P Savoia, A Verrone, M G Bernengo
雑誌名: Dermatology. 1997;194(3):268-72.
Abstract/Text BACKGROUND: Response of cutaneous T-cell lymphoma (CTCL) to systemic chemotherapy is unsatisfactory: despite an initially high response rate (RR), duration is always short-lived.
OBJECTIVE: To investigate the capability of a third-generation regimen including idarubicin in improving RR and response duration in CTCL patients.
METHODS: Twenty-five patients with advanced CTCL (stages IIB and IV) were treated with a 12-week polychemotherapeutic regimen (VICOP-B), which foresees the use of idarubicin in association with etoposide, cyclophosphamide, vincristine, prednisone and bleomycin.
RESULTS: The overall objective RR was 80% (36% complete response). The mycosis fungoides (MF) RR was 84%, with a median duration of 8.7 months. The pleomorphic-lymphoma RR was higher (100%), but the corresponding response duration was shorter (median: 3 months). No responses were documented in Sézary syndrome.
CONCLUSION: VICOP-B regimen is effective and feasible as first-line chemotherapy in advanced MF, with or without extracutaneous involvement.

PMID 9187846  Dermatology. 1997;194(3):268-72.
著者: Herschel S Zackheim, Mohammed Kashani-Sabet, Alex McMillan
雑誌名: J Am Acad Dermatol. 2003 Nov;49(5):873-8. doi: 10.1067/S0190.
Abstract/Text BACKGROUND: Although low-dose methotrexate has been used to treat mycosis fungoides for many years, documentation is very limited.
OBJECTIVE: Our purpose was to review our experience with methotrexate in the treatment of 69 patients with patch/plaque and tumor stage mycosis fungoides observed for up to 201 months.
METHODS: This was a retrospective study. Data are presented in terms of response rates and time to treatment failure.
RESULTS: The greatest number of patients (60) had patch/plaque stage T2 disease (>/=10% skin involved). Of these, 7 (12%) achieved complete remission and 13 (22%) achieved partial remission for a total response rate of 20 of 60 (33%). The median time to treatment failure was 15 months. Only 1 of 7 patients with tumor stage disease responded. Side effects caused treatment failure in 6 (9%) of the total cohort of 69 patients.
CONCLUSION: Low-dose methotrexate may be of value in the treatment of a subset of patients with patch/plaque mycosis fungoides resistant to other therapies.

PMID 14576667  J Am Acad Dermatol. 2003 Nov;49(5):873-8. doi: 10.1067/・・・
著者: H S Zackheim, M Kashani-Sabet, S T Hwang
雑誌名: J Am Acad Dermatol. 1996 Apr;34(4):626-31.
Abstract/Text BACKGROUND: Most patients with erythrodermic cutaneous T-cell lymphoma (CTCL) have intense, generalized, refractory pruritus. In some large series the median survival was approximately 3 years.
OBJECTIVE: Our purpose was to review our experience with methotrexate in the treatment of 29 patients with erythrodermic CTCL observed for up to 129 months.
METHODS: This is a retrospective study. Data are presented in terms of response rates, freedom from treatment failure, and overall survival.
RESULTS: Twelve patients (41%) achieved complete remission, and five (17%) achieved partial remission for a total response rate of 58%. The median freedom from treatment failure was 31 months, and the median survival was 8.4 years. Side effects caused treatment failure in only two patients.
CONCLUSION: Low-dose methotrexate is a valuable first-line treatment for the majority of patients with early to intermediate-stage erythordermic CTCL.

PMID 8601652  J Am Acad Dermatol. 1996 Apr;34(4):626-31.
著者: T Onozuka, K Yokota, T Kawashima, H Shimada, K Kodama, H Kobayashi, H Shimizu
雑誌名: Clin Exp Dermatol. 2004 Jan;29(1):91-2.
Abstract/Text
PMID 14723732  Clin Exp Dermatol. 2004 Jan;29(1):91-2.
著者: P L Zinzani, G Baliva, M Magagnoli, M Bendandi, G Modugno, F Gherlinzoni, G F Orcioni, S Ascani, R Simoni, S A Pileri, S Tura
雑誌名: J Clin Oncol. 2000 Jul;18(13):2603-6.
Abstract/Text PURPOSE: To evaluate the efficacy and toxicity of gemcitabine, a novel pyrimidine antimetabolite with a low-toxicity profile and activity in several solid tumors, in patients with relapsed or refractory cutaneous T-cell lymphomas.
PATIENTS AND METHODS: Between May 1997 and February 1999, 44 previously treated patients with mycosis fungoides (MF; n = 30) and peripheral T-cell lymphoma unspecified (PTCLU) with exclusive skin involvement (n = 14) were enrolled onto a two-institution, phase II trial and treated with gemcitabine. This drug was given on days 1, 8, and 15 of a 28-day schedule at a dose of 1,200 mg/m(2) intravenously over 30 minutes for a total of three courses.
RESULTS: Of the 44 patients, five (11. 5%) achieved complete responses (CRs), 26 (59%) partial responses (PRs), and the remaining 13 showed no benefit from the treatment. Two of the CRs were histologically confirmed. The CR and PR rates were the same for patients with MF and those with PTCLU, respectively. No difference in terms of overall response rate was observed between relapsed and refractory patients. The median durations of CR and PR were 15 months (range, 6 to 22 months) and 10 months (range, 2 to 15 months), respectively. Treatment was well tolerated; hematologic toxicity was mild, and no nausea/vomiting or organ toxicity was recorded.
CONCLUSION: The results of the present phase II study show activity of gemcitabine as a single agent in patients with pretreated cutaneous T-cell lymphoma. Further studies that use gemcitabine alone or in combination with other drugs in earlier stages of the disease are needed.

PMID 10893292  J Clin Oncol. 2000 Jul;18(13):2603-6.
著者: A D Ho, S Suciu, P Stryckmans, F De Cataldo, R Willemze, J Thaler, M Peetermans, H Döhner, G Solbu, M Dardenne, R Zittoun
雑誌名: Ann Oncol. 1999 Dec;10(12):1493-8.
Abstract/Text PURPOSE: Within this phase II EORTC trial, we have investigated the safety and efficacy of pentostatin in lymphoid malignancies. We have previously reported the results in T- and B-cell prolymphocytic leukemia, B-cell chronic lymphocytic leukemia (B-CLL) and hairy cell leukemia. This report focuses on the outcome in T-cell malignancies: T-CLL, Sézary syndrome (Sézary), mycosis fungoides (MF) and T-zone lymphoma (TZL).
PATIENTS AND METHODS: Of the 92 patients with these diagnoses enrolled, 76 were evaluable for response and toxicity, i.e., 25 of 28 with T-CLL, 21 of 26 with Sézary, 22 of 26 with MF, and 8 of 12 with TZL. All patients had progressive and advanced disease. Pentostatin was administered at a dosage of 4 mg/m2 every week for the first 3 weeks, then 4 mg/m2 every 14 days for another 6 weeks, followed by maintenance therapy of 4 mg/m2 monthly for a maximum of 6 months.
RESULTS: Response rates (complete and partial responses) in patients with Sézary (n = 22) or MF (n = 21) were 33% and 23%, respectively, and in patients with T-CLL (n = 21) or TZL (n = 8) 8% and 25%, respectively. Sixteen (21%) patients died during the first ten weeks of treatment: twelve of progressive disease, two of infectious complications with progressive disease, one of myocard infarction and one of renal failure related to administration of i.v. contrast. Major toxicity (grade 3-4) included infection in 11% of patients, nausea/vomiting in 4%, diarrhea in 3%. Hematologic toxicity was mild to non-existent.
CONCLUSIONS: We conclude that pentostatin is active in Sézary and MF but showed marginal activity in T-CLL or TZL. Toxicities are mild to moderate at the dose schedule administered. Due to its relatively specific lympholytic effect and its favorable toxicity spectrum, pentostatin might be especially useful for the palliative treatment of T-cell malignancies.

PMID 10643542  Ann Oncol. 1999 Dec;10(12):1493-8.
著者: Gaëlle Quereux, Sonia Marques, Jean-Michel Nguyen, Christophe Bedane, Michel D'incan, Olivier Dereure, Elisabeth Puzenat, Alain Claudy, Ludovic Martin, Pascal Joly, Michele Delaunay, Marie Beylot-Barry, Pierre Vabres, Philippe Celerier, Bruno Sasolas, Florent Grange, Amir Khammari, Brigitte Dreno
雑誌名: Arch Dermatol. 2008 Jun;144(6):727-33. doi: 10.1001/archderm.144.6.727.
Abstract/Text OBJECTIVE: To assess the rate of objective response to pegylated liposomal doxorubicin hydrochloride (Caelyx) in patients with advanced or refractory cutaneous T-cell lymphoma (CTCL).
DESIGN: Prospective, open, multicenter study.
SETTING: Thirteen dermatology departments in France.
PATIENTS: Twenty-five patients with either (1) stage II to stage IV CTCL previously unsuccessfully treated with at least 2 lines of treatments or (2) histologically transformed epidermotropic CTCL requiring chemotherapy.
INTERVENTION: Administration of Caelyx intravenously once every 4 weeks at a dose of 40 mg/m(2).
MAIN OUTCOME MEASURES: The response to treatment was evaluated by clinical evaluation.
RESULTS: At the end of treatment, we observed an objective response (primary end point) in 56% of the patients (14 of 25): 5 complete responses and 9 partial responses. The median overall survival time was 43.7 months. For the 14 patients who experienced an objective response, the median progression-free survival time after the end of treatment was 5 months.
CONCLUSIONS: This prospective study demonstrates the effectiveness of Caelyx in treating CTCL, with an overall response rate of 56% in spite of the high proportion of patients with advanced-stage disease. Responses were observed in 2 subpopulations of patients in which the prognosis is known to be poorer: Sézary syndrome (overall response rate, 60%) and transformed CTCL (overall response rate, 50%). Moreover, this study shows that dose escalation to 40 mg/m(2) does not seem to improve the effectiveness but increases toxic effects (especially hematologic toxic effects) compared with the dose previously tested of 20 mg/m(2).

PMID 18559761  Arch Dermatol. 2008 Jun;144(6):727-33. doi: 10.1001/arc・・・
著者: G Di Lorenzo, R Di Trolio, M Delfino, S De Placido
雑誌名: Br J Dermatol. 2005 Jul;153(1):183-5. doi: 10.1111/j.1365-2133.2005.06682.x.
Abstract/Text BACKGROUND: Previous studies have shown that pegylated liposomal doxorubicin (LD) is effective in the treatment of relapsing or recalcitrant cutaneous T-cell lymphoma.
OBJECTIVES: To evaluate the activity and toxicity of LD in patients with stage IVB mycosis fungoides (MF).
METHODS: In this retrospective study, we evaluated outcomes and recorded adverse effects in 10 patients with MF (seven men and three women) with extracutaneous involvement. Patients were treated with LD 20 mg m(-2) administered intravenously every 4 weeks.
RESULTS: All patients received at least two cycles of LD, three patients received four cycles and one patient received six cycles. Three patients (30%) had a partial response and two patients had stable disease. Grade 1-2 leucopenia occurred in three of the 10 patients, and grade 4 leucopenia in one. Three patients had grade 2 palmoplantar erythrodysaesthesia.
CONCLUSIONS: This study demonstrates that LD is beneficial in terms of activity and toxicity in stage IVB MF. These observations should be verified in larger studies.

PMID 16029347  Br J Dermatol. 2005 Jul;153(1):183-5. doi: 10.1111/j.13・・・
著者: Werner Kempf, Katrin Pfaltz, Maarten H Vermeer, Antonio Cozzio, Pablo L Ortiz-Romero, Martine Bagot, Elise Olsen, Youn H Kim, Reinhard Dummer, Nicola Pimpinelli, Sean Whittaker, Emmilia Hodak, Lorenzo Cerroni, Emilio Berti, Steve Horwitz, H Miles Prince, Joan Guitart, Teresa Estrach, José A Sanches, Madeleine Duvic, Annamari Ranki, Brigitte Dreno, Sonja Ostheeren-Michaelis, Robert Knobler, Gary Wood, Rein Willemze
雑誌名: Blood. 2011 Oct 13;118(15):4024-35. doi: 10.1182/blood-2011-05-351346. Epub 2011 Aug 12.
Abstract/Text Primary cutaneous CD30(+) lymphoproliferative disorders (CD30(+) LPDs) are the second most common form of cutaneous T-cell lymphomas and include lymphomatoid papulosis and primary cutaneous anaplastic large-cell lymphoma. Despite the anaplastic cytomorphology of tumor cells that suggest an aggressive course, CD30(+) LPDs are characterized by an excellent prognosis. Although a broad spectrum of therapeutic strategies has been reported, these have been limited mostly to small retrospective cohort series or case reports, and only very few prospective controlled or multicenter studies have been performed, which results in a low level of evidence for most therapies. The response rates to treatment, recurrence rates, and outcome have not been analyzed in a systematic review. Moreover, international guidelines for staging and treatment of CD30(+) LPDs have not yet been presented. Based on a literature analysis and discussions, recommendations were elaborated by a multidisciplinary expert panel of the Cutaneous Lymphoma Task Force of the European Organization for Research and Treatment of Cancer, the International Society for Cutaneous Lymphomas, and the United States Cutaneous Lymphoma Consortium. The recommendations represent the state-of-the-art management of CD30(+) LPDs and include definitions for clinical endpoints as well as response criteria for future clinical trials in CD30(+) LPDs.

PMID 21841159  Blood. 2011 Oct 13;118(15):4024-35. doi: 10.1182/blood-・・・
著者: Madeleine Duvic, Michele Donato, Bouthaina Dabaja, Heather Richmond, Lotika Singh, Wei Wei, Sandra Acholonu, Issa Khouri, Richard Champlin, Chitra Hosing
雑誌名: J Clin Oncol. 2010 May 10;28(14):2365-72. doi: 10.1200/JCO.2009.25.8301. Epub 2010 Mar 29.
Abstract/Text PURPOSE: Transformed mycosis fungoides (MF) and Sézary syndrome (SS) are currently incurable. We studied the safety and efficacy of total skin electron beam with allogeneic hematopoietic stem-cell transplantation (HSCT) in patients with cutaneous T-cell lymphoma (CTCL).
PATIENTS AND METHODS: Nineteen patients with advanced CTCL (median age, 50 years; four prior therapies) underwent total skin electron beam radiation followed by allogeneic HSCT between July 2001 and July 2008. Sixteen patients were conditioned with fludarabine (125 mg/m(2)) and melphalan (140 mg/m(2)) plus thymoglobulin (for mismatched donors). Graft-versus-host disease (GVHD) prophylaxis was with tacrolimus/mini methotrexate.
RESULTS: Eighteen patients experienced engraftment, and one died as a result of sepsis on day 16. Median time to recovery of absolute neutrophil count (ANC) was 12 days. Fifteen achieved full donor chimerism, 12 had acute GVHD, and 12 were treated for chronic GVHD. The overall intent-to-treat response was 68%, and the complete response rate was 58%. Four of six patients died in complete remission as a result of bacterial sepsis (n = 2), chronic GVHD and fungal infection (n = 1), or lung cancer (n = 1); only two died as a result of progressive disease. Eight experienced relapse in skin; five regained complete response with reduced immunosuppression or donor lymphocyte infusions. Eleven of 13 are currently in complete remissions, with median follow-up of 19 months (range, 1.3 to 8.3 years). Median overall survival has not been reached.
CONCLUSION: Total skin electron beam followed by allogeneic stem-cell transplantation merits additional evaluation for a selected group of patients with refractory, advanced, cutaneous T-cell lymphoma with evidence for graft-versus-tumor effect.

PMID 20351328  J Clin Oncol. 2010 May 10;28(14):2365-72. doi: 10.1200/・・・
著者: Elise Olsen, Eric Vonderheid, Nicola Pimpinelli, Rein Willemze, Youn Kim, Robert Knobler, Herschel Zackheim, Madeleine Duvic, Teresa Estrach, Stanford Lamberg, Gary Wood, Reinhard Dummer, Annamari Ranki, Gunter Burg, Peter Heald, Mark Pittelkow, Maria-Grazia Bernengo, Wolfram Sterry, Liliane Laroche, Franz Trautinger, Sean Whittaker, ISCL/EORTC
雑誌名: Blood. 2007 Sep 15;110(6):1713-22. doi: 10.1182/blood-2007-03-055749. Epub 2007 May 31.
Abstract/Text The ISCL/EORTC recommends revisions to the Mycosis Fungoides Cooperative Group classification and staging system for cutaneous T-cell lymphoma (CTCL). These revisions are made to incorporate advances related to tumor cell biology and diagnostic techniques as pertains to mycosis fungoides (MF) and Sézary syndrome (SS) since the 1979 publication of the original guidelines, to clarify certain variables that currently impede effective interinstitution and interinvestigator communication and/or the development of standardized clinical trials in MF and SS, and to provide a platform for tracking other variables of potential prognostic significance. Moreover, given the difference in prognosis and clinical characteristics of the non-MF/non-SS subtypes of cutaneous lymphoma, this revision pertains specifically to MF and SS. The evidence supporting the revisions is discussed as well as recommendations for evaluation and staging procedures based on these revisions.

PMID 17540844  Blood. 2007 Sep 15;110(6):1713-22. doi: 10.1182/blood-2・・・

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